Cholesteatoma

Aetiology

• Congenital cholesteatoma: Embryonic cell rest in the middle ear

• Acquired cholesteatoma

  • Primary: Often linked to eustachian tube dysfunction (ETD) causing a retraction pocket in the tympanic membrane (TM) where squamous debris accumulates

  • Secondary: Occurs when skin migrates through a TM perforation, commonly associated with chronic suppurative otitis media

Pathophysiology

• Retraction or invasion of squamous epithelium into the middle ear or mastoid air cells

• Enzymes from keratin debris trigger chronic inflammation and progressive bone erosion (ossicles, mastoid cortex, even facial nerve canal)

Symptoms

• May be asymptomatic initially

• Progressive conductive hearing loss (due to ossicular chain damage)

• Persistent foul-smelling, painless otorrhoea (discharge)

• Chronic middle ear infection or retraction pocket noted on otoscopy

• Possible otalgia, vertigo, or facial nerve weakness if advanced

Differential Diagnosis

• Chronic otitis media with effusion

• Otosclerosis

• Tumours of the external ear canal or middle ear

• Simple tympanic membrane perforations

• Benign ear masses (e.g. polyp)

Investigations

Otoscopy

• Retraction pocket, visible white/pearly mass or granulation tissue

• Typically found in the posterosuperior quadrant of the TM

• “Unsafe” perforation signs (granulation tissue, bone erosion) are highly suspicious

Audiometry

• Conductive hearing loss most common

• Mixed or sensorineural loss if labyrinth or cochlea involved

Imaging

• High-resolution CT (Temporal Bone) to assess extent of bone erosion, mastoid involvement, and any complications

• MRI may help if intracranial extension or complications are suspected

Management

Surgical (Definitive)

• Mastoidectomy or tympanomastoid surgery to remove the cholesteatoma, prevent complications, and restore/maintain hearing

• Ossicular chain reconstruction may be needed if there is ossicle destruction

Conservative/Supportive

• For patients unfit for surgery or awaiting surgical management

• Keep the ear dry (use earplugs during bathing)

• Aural toilet under microscopic guidance to remove debris

• Topical or oral antibiotics for acute infections

• Regular ENT follow-up to monitor disease progression

Complications

Hearing Loss

• Usually conductive from ossicular erosion, though can be sensorineural if the inner ear is involved

Intracranial Infections

• Abscess, meningitis, or sigmoid sinus thrombosis if infection spreads

Vertigo

• Signals labyrinthine involvement

Facial Nerve Paralysis

• Occurs if the facial nerve canal is eroded

Additional Notes

• Early recognition of cholesteatoma is crucial to avoid destructive complications

• Persistent otorrhoea with an attic perforation or retraction pocket is considered “unsafe” and warrants urgent ENT referral

• Postoperative follow-up is essential as cholesteatoma can recur

• Mastoid bowl management (if created during surgery) involves regular cleaning to prevent re-accumulation of debris or infection

• High suspicion warranted in patients with chronic ear infections not responding to standard treatment, especially when foul-smelling discharge and hearing loss are present